Abstract:
The presence of interleukin-2 (IL-2) and gamma-interferon (gamma IFN) was measured by conventional tests after mitogen stimulation of peripheral blood mononuclear cells (PBMCs) of patients who had immunodeficiencies and severely defective T lymphocyte functions in vivo and in vitro. Based on CDC-NIH criteria, 20 of the patients had common variable immunodeficiency (CVI); 3, severe combined immunodeficiency (SCID); 3, Wiskott-Aldrich (WA) syndrome; 3, ataxia-telangiectasia (AT); 2, hyper IgE syndrome; 1, combined immunodeficiency (CID) with hyper IgM; and 12, lymphadenopathy syndrome (LAS). Total T lymphocytes, identified by OKT3 reactivity, were low in the patients who had SCID, CID with hyper IgM, AT, WA syndrome, or hyper IgE syndrome. Variable levels of the T lymphocytes were found in the patients who had CVI (51-84%) or LAS (46-81%), but all levels were within 2 SD of the normal mean. Low or inverted OKT4/OKT8 ratios were found in 15/20 CVI patients, and an inverted OKT4/OKT8 ratio with absolute decrease of T4+ lymphocytes was detected in 9/12 LAS patients. In vitro response to phytohemagglutinin (PHA) of PBMCs of patients with impaired cell-mediated immunity (CMI) was grossly abnormal, with cpm values below 10,000, except for four patients who showed some degree of lymphoproliferation. IL-2 production by PHA-stimulated PBMCs was severely depressed in the patients who had SCID, CID with hyper IgM, AT, WA syndrome, or hyper IgE syndrome; IL-2 production after PHA induction was measured in only six of the LAS patients, and normal levels were found in all six. IL-2 receptor (Tac) expression in the CMI deficient patients was lower than that in normal subjects. Major defects in gamma IFN production were lacking in most patients who had CVI or LAS; in contrast, patients with severe T cell defects had grossly defective or impaired production of gamma IFN. PBMCs of most SCID patients showed no response to added IL-2; 2/3 AT patients, 3/3 WA patients, and 2/2 hyper IgE patients similarly showed no responses when IL-2 was added to their PBMCs. (13 Refs)
Keywords: Antigens, Surface/IMMUNOLOGY AIDS-Related Complex/IMMUNOLOGY Human Immunologic Deficiency Syndromes/*IMMUNOLOGY Interferon Type I/BIOSYNTHESIS Interferon Type II/*BIOSYNTHESIS Interleukin-2/*BIOSYNTHESIS Lymphocyte Transformation Phytohemagglutinins/IMMUNOLOGY Receptors, Immunologic/IMMUNOLOGY T-Lymphocytes/*IMMUNOLOGY T-Lymphocytes, Helper-Inducer/IMMUNOLOGY T-Lymphocytes, Suppressor-Effector/IMMUNOLOGY MEETING PAPER
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